Interatrial septal aneurysm and genetic prothrombotic disorders: possible interaction in the pathogenesis of pediatric stroke.

Stroke in children is a rare condition and has a multifactorial etiology. The association between ischemic stroke in young adults and some minor cardiac abnormalities such as atrial septal aneurysm with or without interatrial shunting has recently been reported: however, the pathogenetic mechanism still remains unclear. Genetic and acquired prothrombotic disorders are also risk factors for cerebral ischemic events in children. We report a case of ischemic stroke in a 10-year-old female child who was heterozygous for the prothrombin G20210A variant and who presented with an atrial septal aneurysm associated with an interatrial shunt. We hypothesize that these risk factors play a synergic role but their relative importance and whether alone they can determine cerebral embolism remain to be determined.

[The complete regression of multiple cardiac rhabdomyomas in childhood]. / Regressione completa di rabdomiomi cardiaci multipli in età pediatrica.

Rhabdomyoma, even if rare, is the most common primary cardiac tumor in infancy and childhood. We present the case of a newborn in whom several intramural and intracavitary cardiac masses were found at birth by two-dimensional echocardiography, and showed complete regression at the follow-up examinations. Except for supraventricular arrhythmias, there were no symptoms or signs of cardiac involvement at birth: the baby was acyanotic and is no apparent distress; no murmur was detected and chest roentgenogram revealed normal heart size; association with Tuberous Sclerosis was excluded. Despite major anatomical cardiac anomaly, neither in-flow nor out-flow tract obstruction was found by Doppler investigation and therefore a noninterventional approach was preferred. At the two-year follow-up control the baby was asymptomatic and the echocardiogram revealed complete regression of cardiac tumors. Our report confirms the possible favourable outcome of Rhabdomyoma and the utility of echocardiography in these patients' follow-up.

[A case of transient hypertrophic cardiomyopathy in a twin infant of a diabetic mother]. / Un caso di cardiomiopatia ipertrofica transitoria in neonata gemella figlia di madre diabetica.

The case is reported of a dizygotic twin infant of a diabetic mother with transient hypertrophic cardiomyopathy. The clinical course and eco-ecg reports are described. The etiopathogenesis is discussed since no evidence of cardiomyopathy was found in the second twin. The importance of a timely diagnosis for a correct therapy is emphasized.

The relationship between growth of atherosclerotic plaques, variant angina and sudden death.

Clinico-pathological findings are described in two patients with typical variant angina who died suddenly during an ischemic attack. In both cases, detailed pathologic examination of the coronary arteries disclosed severe focal atherosclerosis of the anterior descending coronary artery. The only distinctive histological finding was new intimal proliferation of smooth muscle cells enmeshed within mucoid substance, superimposed on the old fibrous cap of the plaque. These findings agree with experimental and clinical data which suggest that coronary vasospasm may be related to growth of atherosclerotic plaques. This study provides histological evidence that progression of an atherosclerotic plaque may underlie variant angina and sudden death.